Symptoms

The major symptom is prolonged massive watery diarrhea. People produce 1 to 3 quarts (1,000 to 3,000 milliliters) of stool per day, causing dehydration. In 50% of people, diarrhea is constant, and in the rest, the severity of the diarrhea varies over time.

Because the diarrhea removes many of the body’s normal salts, people often develop low blood levels of potassium ( hypokalemia) and excessively acidic blood ( acidosis). These changes can cause lethargy, muscular weakness, nausea, vomiting, and crampy abdominal pain. Some people have flushing.

Diagnosis

Blood tests

Imaging tests

A doctor bases the diagnosis on the person’s diarrhea symptoms and finding elevated levels of vasoactive intestinal peptide (VIP) in the blood.

People with elevated levels of VIP should also have the imaging tests endoscopic ultrasonography (see Ultrasound Scanning (Ultrasonography)), positron emission tomography (PET), and octreotide scintigraphy or arteriography (an x-ray taken after a radiopaque dye is injected into an artery) to detect the location of the vipoma.

Treatment

Replacement of fluids and electrolytes

Octreotide

Surgical removal

Initially fluids and electrolytes (minerals in the blood such as potassium and sodium) must be replaced by vein (intravenously). Bicarbonate must be given to replace that lost in the stool and to prevent acidosis. Because water and electrolytes continue to be lost in the stool as rehydration is achieved, doctors may find it difficult to continually replace water and electrolytes.

The drug octreotide usually controls diarrhea, but large doses may be needed.

Surgical removal of the vipoma cures about 50% of people whose tumor has not spread. Surgery may temporarily relieve symptoms in people whose tumor has spread. Chemotherapy drugs may reduce diarrhea and the size of the tumor but do not cure the disease.

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